1. Chapter 12
Cardiac Disorders

2. Cardiovascular System
Heart fully functioning by 8th week of gestation
Fetal circulation: placenta is the organ of oxygenation
Ductus venosus
Ductus arteriosus
Foramen ovale
The birthing process begins closure of fetal shunts
Heart defects are the most common birth defect
Leading cause of birth defect-related deaths
The heart is a vital organ; parents confronted with a child’s cardiac problems are overwhelmed with fear and anxiety.

3. Congenital Heart Disease
Description
A defect in the structure of the heart or in one or more of the large blood vessels that lead to and from the heart
Multifactorial
Result of genetic-environmental interactions
Four classifications
Based on defect’s effect on blood flow
Hemodynamics: study of blood circulation
Shunting: flow of blood through abnormal openings
Defects with increased pulmonary blood flow
Obstructive defects
Defects with decreased pulmonary blood flow
Mixed defects
The rate of incidence of infants born with a congenital heart defect is about one per 125 to 150 deliveries.
Genetic factors: History of CHD, chromosomal abnormalities
Environmental factors: Alcoholism, cocaine, rubella, exposure to Coxsackie virus, diabetes mellitus, ingestion of lithium salts, Accutane, advanced maternal age

5. Congenital Heart Disease (CHD)
Diagnostic tools
Not all testing is necessary for each child
Lab tests, electrocardiogram, halter monitor, event recorder, chest radiography, echocardiogram, MRI, cardiac catherization
Cardiac catherization is an invasive procedure
Provides information about anatomy, cardiac pressure, oxygen saturation, cardiac function
Sedation is necessary
Entry site kept straight 4-6 hours after procedure

6. Congenital Heart Disease (CHD)
Signs and symptoms
Depend on location and type of heart defect
Child may be small for age, condition may be classified as a physiologic failure to thrive
Exercise intolerance noticed anywhere from infancy to toddler age
Clubbing of the fingers
Frequent respiratory infections because of pulmonary vascular congestion
Squatting position (TET spell)
Polycythemia: Body compensates for hypoxemia by increasing number of RBCs
Why do some children with CHD frequently assume a squatting position?

7. Clubbed fingers

8. Congestive Heart Failure (CHF)
Early symptoms
Lack of infant weight gain
Progressive symptoms
Difficulty feeding/ sucking due to air hunger
Periorbital/facial edema
Vein distention
Infant irritability and fatigue
Hepatomegaly
Splenomegaly
Tachycardia/tachypnea
Decreased urine output
Increased work of breathing
Diaphoresis
Mottling
Cyanosis
Pallor
CHF is not a disease, but rather symptoms caused by an underlying heart defect.

9. Defects with Increased Pulmonary Blood Flow
Patent ductus arteriosus (PDA)
Passageway connecting pulmonary artery to aorta, avoiding fetal lungs
Failure to close causes oxygenated blood to recycle through the lungs
Overburdens pulmonary circulation
Makes heart work harder
One of the most common cardiac anomalies
Symptoms
Machine-like murmur, dyspnea, bounding pulses on exertion, failure to thrive, frequent respiratory infections
Treatment
Indomethacin/ibuprofen in premature infants to close ductus
Amplatzer PDA device
Surgical repair
Symptoms of patent ductus arteriosus may go unnoticed during infancy.
PDA occurs twice as frequently in females.
Excellent prognosis for corrected PDA.

10. Defects with Increased Pulmonary Blood Flow
Atrial septal defect (ASD)
Abnormal opening between right and left atria
Common congenital heart anomaly
Symptoms
Generally asymptomatic
Cyanosis if blood flow is reversed by heart failure
Large openings may cause failure to thrive
Treatment
Open heart surgery
Percutaneous occluding devices
ASD more common in females.
Both surgical and nonsurgical procedures have good results.

11. Defects with Increased Pulmonary Blood Flow
Ventricular septal defect (VSD)
Opening between right and left ventricles
75% of small VSDs close spontaneously by age 10
Symptoms
Loud, harsh murmur
Systolic tremor
Moderate/large defects may present CHF symptoms
Treatment
Percutaneous transcatheter closure
Occluder devices

12. Obstructive Defect Coarctation (tightening) of the aorta Symptoms
Increased pressure proximal to the defect
Decreased pressure distal to the defect
High blood pressure
CHF symptoms
Treatment
Balloon angioplasty
Surgical intervention; anastomosis
Risk of developing subacute bacterial endocarditis
High risk of recurrence for repairs done in infancy.

13. Defect with Decreased Pulmonary Blood Flow
Tetralogy of Fallot
The most common cyanotic heart defect
Four defects
Stenosis (narrowing of pulmonary artery)
Hypertrophy of the right ventricle
Over-riding aorta
VSD
Symptoms
Cyanosis/hypoxemia episodes with hyperpnea, irritability
Hypoxia
Treatment
Open heart surgery to correct defects
Hypoxia triggered when there is an acute demand for oxygen; i.e., during blood draw, defecation, feeding, crying.
How should spells of hypoxia be handled?
What is given to stop hyperpnea?

14. Mixed Defect Transposition of the great arteries (TGA)
Pulmonary artery leaves left ventricle
Aorta leaves the right ventricle
Other defects (septal defects, PDA) must be present to exchange blood which sustains life
Survival impossible without surgery
Symptoms
CHF symptoms
Any murmur present is caused by other defects, not TGA
Treatment
Prostaglandin E1 (keeps PDA open)
Balloon atrial septotomy (opens septal wall)
Corrective surgery
Performed within the first 2 weeks of life
Prostaglandin E1 and balloon atrial septostomy are used as palliative measures until corrective surgery.
Surgery involves switching the arteries.
Antibiotic prophylaxis is done for the first 6 months.

15. Mixed Defect Hypoplastic left heart syndrome (HLHS)
Left side of the heart is underdeveloped
Hypoplasia of aorta, left ventricle, mitral valve
Systemic circulation provided by right side of the heart (Rt ventricle is force of circulation)
Ductus arteriosus and foramen ovale must remain patent to survive with HLHS
Treatment
PGE1 is administered to maintain a PDA
Surgery in several stages
Norwood, Glenn, Fontan
Transplants have been successful, but donor availability, organ rejection, infection, and immunosuppression are complicating factors.

16. Congenital Heart Disease
Treatment and nursing care
Nursing goals in the care of the newborn infant can be adapted for all children with heart defects
Reduce the work of the heart
Improve respiration
Maintain proper nutrition
Prevent infection
Reduce the anxiety of the patient
Support and instruct the parents
Why is Lanoxin the preferred oral digitalis preparation for children with CHD?
The parents of the child need support and understanding over a long period.
Why is cerebral thrombosis a concern?

17. Congenital Heart Disease
Treatment and nursing care (continued)
Change child’s position frequently to prevent respiratory complications
Threat of cerebral thrombosis
Chest tubes may be used after surgery
System must be airtight
Drainage containers always kept below the level of the chest
Avoid unnecessarily disturbing the child; they need to conserve energy
Common medications
Digoxin (Lanoxin)
Dopamine, dobutamine, epinephrine
Amrinone, milrinone
ACE inhibitors
Angiotensin II receptor blockers
Diuretics
How should the nurse measure pulse before medicating with Lanoxin?

18. Congenital Heart Disease
Treatment and nursing care (continued)
Infective endocarditis (IE)
High risk for children with complex cyanotic heart diseases or children who have had heart surgery
Organisms grow on the endocardium or areas of turbulent blood flow
Symptoms
Fever, fatigue, headache, nausea, vomiting
Diagnosis and treatment
Blood cultures determine causative organism
Antibiotics

19. Congenital Heart Disease
Home care
Family must understand medication administration
Family must identify symptoms requiring medical attention
Provide a normal environment within child’s limits
Avoid allowing the child to gain control of the home
Limit setting
Integrate the child into family life
Explain the possible need for frequent hospitalization to parents and child
Avoid rough play for ~6 weeks.
All day attendance in school may be too tiring for some conditions.

20. Question 12.2
Which of the following is NOT one of the four classifications of congenital heart disease?
Defects with increased pulmonary blood flow
Congestive heart failure
Defects with decreased pulmonary blood flow
Mixed defects
Answer: B

21. Acute Rheumatic Fever Description Signs and symptoms
Follows infection with certain strains of Group A beta-hemolytic streptococci
Signs and symptoms
Abdominal pain, fever, pallor, fatigue, anorexia, unexplained nosebleeds
Jones criteria aid in diagnosis of rheumatic fever
ECG is sometimes a useful diagnostic
When does rheumatic fever typically occur?
Rheumatic fever has a tendency to recur, and each attack carries the threat of further damage to the heart.
Signs and symptoms of rheumatic fever:
Range from mild to severe
May not occur for several weeks after a streptococcal infection

24. Acute Rheumatic Fever Treatment and nursing care
Elimination of the initial infection is followed by long-term chemo-prophylaxis (prevention of disease by drugs)
Intramuscular penicillin G benzathine (Bicillin), given as an intramuscular injection every 28 days
Anti-inflammatory drugs are used to decrease pain and inflammation

25. Acute Rheumatic Fever Home care
Bed rest during the initial attack is not necessary but is recommended if carditis is present
Nurse must verify that parent and child understand activity limitations
Parents should provide interesting quiet activities
Long-term chemo-prophylaxis

26. Acute Rheumatic Fever Prevention
The nurse is involved in prevention of rheumatic fever in the community by recognizing signs and symptoms of streptococcal infections, doing screening, and referring for treatment